Download full-text PDF

Source
http://dx.doi.org/10.1016/0014-5793(75)80254-7DOI Listing

Publication Analysis

Top Keywords

debranching enzyme
4
enzyme rabbit
4
rabbit skeletal
4
skeletal muscle
4
muscle evidence
4
evidence location
4
location active
4
active centres
4
centres single
4
single polypeptide
4

Similar Publications

Plant chloroplasts store starch during the day, which acts as a source of carbohydrates and energy at night. Starch granule initiation relies on the elongation of malto-oligosaccharide primers. In Arabidopsis thaliana, PROTEIN TARGETING TO STARCH 2 (PTST2) and STARCH SYNTHASE 4 (SS4) are essential for the selective binding and elongation of malto-oligosaccharide primers, respectively, and very few granules are initiated in their absence.

View Article and Find Full Text PDF

Unlabelled: Recent studies report the genetic loss of the lariat debranching enzyme ( ) activity increases susceptibility to viral infection. Here, we show that more than 25% of human introns contain large hairpin structures created by the folding of two elements inserted in opposite orientation. In wildtype cells, this large reservoir of endogenous dsRNA is efficiently degraded.

View Article and Find Full Text PDF

Background: High resistant starch (RS) rice with a low glycemic index (GI) is digested more slowly, resulting in a slow increase in the postprandial blood glucose level. People with milled rice as a staple diet, and with sedentary lifestyles, are more prone to acquire type 2 diabetes in the long term. With an increasing population of diabetics worldwide, one possible solution is the development of rice-based, low-GI foods with high RS content rice.

View Article and Find Full Text PDF

Human DBR1 deficiency impairs stress granule-dependent PKR antiviral immunity.

J Exp Med

January 2025

Division Life Sciences and Medicine, Department of General Surgery, The First Affiliated Hospital of USTC, Key Laboratory of Immune Response and Immunotherapy, Center Advanced Interdisciplinary Science and Biomedicine IHM, University of Science and Technology of China, Hefei, China.

The molecular mechanism by which inborn errors of the human RNA lariat-debranching enzyme 1 (DBR1) underlie brainstem viral encephalitis is unknown. We show here that the accumulation of RNA lariats in human DBR1-deficient cells interferes with stress granule (SG) assembly, promoting the proteasome degradation of at least G3BP1 and G3BP2, two key components of SGs. In turn, impaired assembly of SGs, which normally recruit PKR, impairs PKR activation and activity against viruses, including HSV-1.

View Article and Find Full Text PDF

[Genetic and clinical characteristics of 26 cases with glycogen storage disease type Ⅲ].

Zhonghua Gan Zang Bing Za Zhi

November 2024

The Center for Pediatric Liver Diseases, Children's Hospital of Fudan University, Shanghai201102, China.

Article Synopsis
  • The study analyzed the genetic and clinical profiles of 26 patients with glycogen storage disease type III (GSD III), focusing on the effects of different genetic mutations in the AGL gene.
  • It found that the most common symptoms included elevated liver enzymes, hepatomegaly, and fasting hypoglycemia, and that liver biopsies revealed significant fibrosis in 83.3% of cases.
  • After treatment with uncooked cornstarch for over a year, patients showed improvements in growth, liver size, and metabolic markers, although a subset of cases still experienced clinical issues.
View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!